title: Electroretinographic findings in macular dystrophy.
abstract: The flash and pattern electroretinogram were investigated in a group of families with rare forms of inherited macular dystrophy, which included Sorsby's fundus dystrophy, X-linked retinoschisis and macular dystrophy of uncertain classification and variable expression. Flash electroretinograms, under both photopic and scotopic conditions, were attenuated in both Sorsby's fundus dystrophy and X-linked retinoschisis--with some effect on implicit time being noted in the latter condition--but in the unknown group the effect was less demonstrable, only 50% having attenuated flash electroretinograms. Pattern electroretinograms were reduced in all three conditions and in almost all cases. The study demonstrates that some so-called macular dystrophies also have widespread abnormalities affecting the peripheral retina. These findings may contribute to a better understanding of the underlying pathophysiologic mechanisms in these rare forms of retinal dysfunction.
authors.affiliation: University Department of Ophthalmology, School of Neurosciences, Faculty of Medicine, University of Newcastle upon Tyne, UK.
authors.forename: M P
authors.initials: MP
authors.lastname: Clarke
authors.forename: K W
authors.initials: KW
authors.lastname: Mitchell
authors.forename: S
authors.initials: S
authors.lastname: McDonnell
mesh_terms: D000328:Adult; D000368:Aged; D002648:Child; D004596:Electroretinography; D005260:Female; D008040:Genetic Linkage / Q000235:genetics; D006801:Humans; D008266:Macula Lutea / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D010775:Photic Stimulation / Q000379:methods; D012164:Retinal Diseases / Q000503:physiopathology; D012167:Retinal Perforations / Q000235:genetics* / Q000503:physiopathology; D014960:X Chromosome